Melorheostosis is a rare, noninheritable developmental dysplasia of cortical bone that is often characterized by a classic radiographic pattern of flowing hyperostosis along the cortex of long bones1. Patients with this condition may have joint pain, stiffness, deformity, and restricted range of motion due to soft-tissue contracture2. Involvement usually follows a sclerotomal distribution3 and usually affects only one extremity4,5. Joint contracture occurs when the abnormal ossification in the cortex of the long bone involves the soft tissues and extends into the joint, resulting in soft-tissue fibrosis and contracture2,5,6. There is no specific treatment for this condition; options range from nonsurgical management (e.g., splinting and early training in making optimal use of the unaffected extremity) to various types of surgical management (e.g., tendon lengthening, sympathectomy, implant arthroplasty, or even amputation2. The literature indicates that surgical release of a joint contracture may be difficult and that recurrence of the deformity is frequent2,4-6.
Melorheostosis of the elbow is rare compared with that of the lower extremity, and a thorough search of the literature revealed no reports on surgical release of a stiff elbow caused by this condition. We report the case of a thirty-seven-year-old man who had a fixed flexion contracture of the elbow as a result of isolated melorheostotic involvement of the proximal part of the ulna and who obtained a functional range of motion after open release of the elbow. The patient was informed that data concerning the case would be submitted for publication, and he consented.
A thirty-seven-year-old man presented with limitation of motion of the left, nondominant elbow. He reported that pain and limited elbow motion had developed after he played a game of squash five years previously. There was no history of elbow trauma or infection. The pain had decreased with time, but limitation of elbow motion had continued to increase and had resulted in a fixed flexion deformity of 90° two years before the patient presented to us. Physical examination revealed no decrease in the range of supination and pronation of the left forearm when compared with that of the right. The patient had no ulnar nerve symptoms; sensibility in the ulnar nerve distribution was normal, and there was no intrinsic atrophy or clawing of the hand.
Plain radiographs of the affected elbow showed cortical hyperostosis of the proximal part of the ulna and speckled calcification of the distal insertion of the triceps (Fig. 1). A computed tomographic scan revealed nodularity of the periosteal bone formation and the presence of a flowing candle-wax pattern on a short segment of bone (Fig. 2). Magnetic resonance imaging demonstrated low signal intensity in all sequences (a classic sign of bone changes) and synovial inflammation without joint destruction or muscle atrophy (Fig. 3). A diagnosis of melorheostosis of the proximal part of the ulna was made on the basis of the findings from these imaging studies.
Because splinting and physical therapy had proven to be ineffective and because the patient had severe limitation of functional use of the extremity, we recommended surgical release of the contracture.
The procedure was performed through a medial approach to the elbow7,8. The ulnar nerve was identified and mobilized. There were no adhesions along the ulnar nerve. With care taken to preserve the anterior oblique bundle of the medial collateral ligament, we resected all medial fibrotic, contracted capsuloligamentous structures. At this point, passive range of motion of the elbow was from 20° of flexion to 110° of flexion. The dissection was extended posteriorly through the interval between the triceps and the posterior aspect of the humerus. Contracted fibrous tissue was removed along with the calcification in the area of the triceps insertion. There was synovial thickening in the olecranon fossa, but the osseous contour was intact. We obtained 140° of elbow flexion without further release of the triceps fascia or muscle. Because extension was still limited, an anterior release was performed. The flexor-pronator muscle mass was found to be contracted, therefore necessitating release of the common origin of these muscles by Z-plasty. To achieve full extension, the brachialis muscle was elevated from the anterior aspect of the humerus and the anterior part of the elbow capsule was resected under direct visualization. After hemostasis was obtained, the flexor-pronator muscle was repaired in a lengthened position and the ulnar nerve was transposed subcutaneously. The wound was closed over suction drains, and the elbow was immobilized in an extended position.
The hyperostotic cortical lesion of the proximal part of the ulna was partially excised for tissue diagnosis during the operation. Histologic examination of the tissue revealed dense bone, which was consistent with the diagnosis of melorheostosis (Fig. 4).
Three days postoperatively, the elbow was mobilized with use of a continuous-passive-motion machine. The anterior cubital area developed a large bulla, which stabilized and resolved. The patient was discharged seven days postoperatively with a passive range of motion from 20° of flexion to 100° of flexion. Under the supervision of a therapist, gentle range of motion was encouraged for the next six weeks. An extension splint was worn at night during this time. Two years postoperatively, the range of motion was 20° of flexion to 135° of flexion and there was no sign of recurrence of the contracture (Fig. 5).